The development of chronic kidney disease (CKD) was evaluated in a large cohort of pediatric lung transplant recipients. Retrospective chart review identified 125 patients undergoing first lung transplant at St. Louis Children's Hospital and surviving 1 year. Mean age at transplant was 10.3 +/- 0.55 years, while mean time after transplant was 4.9 years. Serum creatinine nearly doubled from baseline 0.48 mg/dL +/- 0.02 (n = 125) to 0.87 mg/dL +/- 0.04 (n = 120) at 1 year, and tripled to 1.39 mg/dL +/- 0.15 (n = 23) by 7 years after transplant. The glomerular filtration rate (GFR), as estimated by the Schwartz formula, decreased from baseline 163 +/- 5.9 mL/min/1.73 m(2) (n = 109) to 88 +/- 2.5 (n = 104), reaching 69 +/- 9.0 (n = 6) by 10 years (p < 0.01). Seven patients developed end-stage kidney disease, and by 5 years after transplant, 38% of patients reached GFR < 60 mL/min. Older age at transplant and primary diagnosis of cystic fibrosis (CF) were both associated with decreased renal survival by Kaplan-Meier (KM) analysis. In summary, pediatric lung transplant recipients experience significant loss of renal function over time, as observed in other solid organ transplant recipients, and is most dramatic in adolescents.